A case of clear cell carcinoma arising in endometriosis of the retroperitoneum is presented. It occurred 5 years after supracervical hysterectomy and bilateral salpingo-oophorectomy and following 4 years of estrogen therapy. The literature concerning malignancy developing in extragonadal endometriosis has been reviewed and is summarized as follows: 1) 45 cases have been compiled, of which 32 were carcinomas and 13 sarcomas; 2) adenocarcinoma was the most common histologic type, although virtually every tumor of mullerian derivation has been described; 3) the typical patient was nulliparous and perimenopausal; 4) the rectovaginal septum was the most common site, and in general the frequency of malignancy i n a given site parallels the frequency of endometriosis in that location; 5 ) simultaneous tumors of the uterus or ovary were present i n seven cases (15%); 6) prognosis appeared affected by site and histologic type; 7) a history of prior pelvic irradiation to effect castration was present in three (9%) of the patients with adenocarcinoma; and 8) four (12%) of the patients with adenocarcinoma were subjected to exogenous estrogens or estrogen-secreting ovarian tumors.CUWW 40:3065-3073, 1977.
NDOMETRIOSIS, ALTHOUGH IT OCCURS MOSTE frequently in the pelvic organs, has been found rarely in such distant sites as the lungs,' lymph nodes, " ureter,'.87 and kidney. 47 This ectopic endometrial tissue may undergo cyclical histologic changes secondary to hormonal influences. Just 3 years after he described endometriosis as an entity, Sampson" reported the first cases of malignancy arising in ovarian endometriosis. Occasional reports have appeared implicating adenomyosis as the origin of neoplasia, occasionally simultaneous with endometrial carcinoma. 16.17,10.28,80,63. 75 Malignant change has also'been known to occur in extragonadal endometriosis, and the presentation of such a case at our institution prompted a review of the literature on this subject.
3065CASE REPORT M.A., a 48-year-old nulliparous white woman, was admitted to the Hospital of the University of Pennsylvania on September 16, 1975. Two months prior to this admission she began to notice mild nonradiating right-sided lower abdominal pain, urinary frequency, constipation, but no vaginal discharge or other symptoms. Past medical and gynecologic history revealed that the patient had complaints of dysmenorrhea for years. A dilation and curettage performed in 1970 showed cystic hyperplasia. In June, 1971 the patient underwent a supracervical hysterectomy and bilateral salpingo-oophorectomy for dysfunctional bleeding. Pelvic endometriosis and multiple abdominal adhesions were encountered by the surgeon. These adhesions were severe in the rectovaginal pouch and necessitated the supracervical approach. Gross pathologic examination of the specimen (HUP #B75726) disclosed a 375-g uterus, markedly distorted by multiple leiomyomas. Microscopic sections revealed a pregnant uterus, a corpus luteum of pregnancy in the right ovary, adenomyosis, and left ovarian endometri...